Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome.
Horm Res Paediatr
; 88(3-4): 285-290, 2017.
Article
de En
| MEDLINE
| ID: mdl-28528327
ABSTRACT
BACKGROUND:
Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS.OBJECTIVE:
A 6-month-old girl presented with café-au-lait spots, short stature, central obesity, a moon face, and hypertension. Endocrinological tests and imaging studies led to the diagnosis of ACTH-independent Cushing syndrome due to bilateral adrenal hyperplasia induced by MAS. "Three-quarters adrenalectomy", namely right-sided total adrenalectomy and left-sided half adrenalectomy, was carried out. An activating mutation of the GNAS1 gene (p.Arg201Cys) was identified in the adrenal tissues. Since the operation, our patient has been in a state of clinical remission for more than 2 years.CONCLUSION:
Our original surgical intervention, three-quarters adrenalectomy, may be a new treatment option for Cushing syndrome associated with MAS.Mots clés
Texte intégral:
1
Collection:
01-internacional
Base de données:
MEDLINE
Sujet principal:
Hyperplasie congénitale des surrénales
/
Surrénalectomie
/
Syndrome de Cushing
/
Dysplasie fibreuse polyostotique
Type d'étude:
Etiology_studies
/
Prognostic_studies
Limites:
Female
/
Humans
/
Infant
Langue:
En
Journal:
Horm Res Paediatr
Sujet du journal:
ENDOCRINOLOGIA
/
PEDIATRIA
Année:
2017
Type de document:
Article
Pays d'affiliation:
Japon